NF2関連神経鞘腫症（NF2SWN)のマネジメント

Management of Neurofibromatosis Type 2

Point

・NF2関連神経鞘腫症の聴神経腫瘍に対する手術・放射線治療はともに，一般の聴神経腫瘍と比べて治療成績が劣り，聴力温存が困難な場合が多い．

・両側の高度難聴症例に対しては人工聴覚器による聴覚再建を検討するが，蝸牛神経温存例においては人工内耳が有効であり，これを念頭に置いた治療選択が重要である．

・今後，ベバシズマブによる薬物療法，血管内皮成長因子（VEGF）受容体に対するワクチン治療の実用化が期待される．

　Surgical and radiotherapeutic interventions for vestibular schwannomas in patients with neurofibromatosis type 2 (NF2) generally yield less favorable outcomes than in sporadic unilateral cases, often hindering hearing preservation. Managing NF2 entails a lifelong series of complex decisions, repeatedly weighing functional preservation against impairment and surveillance against invasive therapeutic interventions. For both patients and their physicians, a critical issue lies in how to maximize functional preservation while simultaneously securing long-term survival. Auditory reconstruction using implantable auditory devices should be considered in cases of severe bilateral hearing loss. In patients with preserved cochlear nerve integrity, cochlear implantation is effective, and treatment strategies should be planned with this option in mind. Clinical trials of bevacizumab for pharmacological treatment are currently ongoing in Japan, and the clinical application of anti-vascular endothelial growth factor receptor vaccine therapy is anticipated.